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Query: UMLS:C0018799 (heart disease)
 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Churg-Strauss syndrome (CSS) is a rare systemic disease characterized by necrotizing vasculitis and peripheral eosinophilia. Cardiac involvement is considered common and is given a high rank among the causes of morbidity and mortality. The aim of this study was an update on the cardiac manifestations of this syndrome using a noninvasive approach. Sixteen patients with CSS were compared with a gender- and age-matched group of 20 healthy subjects. All patients but 1 were receiving treatment (steroids and/or immunosuppressive drugs). According to the Birmingham vasculitis activity score, 12 patients were in an active phase, and 4 were in drug-induced remission. All subjects underwent M-B-mode echocardiography and Doppler tissue echocardiography. Heart failure, life-threatening arrhythmias, and other prominent manifestations of heart disease were not observed. No differences were found in left ventricular diameter, volume, mass, or ejection fraction. The 2 groups did not differ in right ventricular diameter and pulmonary pressure. Few and nonspecific changes were detected by 2-dimensional echocardiography, including subclinical pericardial effusion and mitral regurgitation, in fewer than half the subjects. Subjects with CSS showed an impairment of ventricular relaxation. Changes were more prominent in the right ventricle. The peak velocity (PV) of early diastolic tricuspid inflow (E) was about 8% less than in controls, and the velocity of late diastolic inflow (A) was 35% greater. The E/A(PV) ratio was, on average, 33% less. In the left ventricle, E(PV) was 11% less and A(PV) 11% greater. The E/A ratio was decreased by 22%. Doppler analysis of tissue kinetics confirmed these indications. In the right ventricle, E(PV) was decreased by 10% and A(PV) was increased by 20% in the patient group. The E/A(PV) ratio was decreased by 29%. In the left ventricle, in which different sites were sampled, the average changes were -15%, +1%, and -23%, respectively. In the left ventricle, the velocity of systolic contraction was also decreased by 12%. Because of the small group size, only some of these differences were statistically significant. In conclusion, these moderate changes, devoid of clinical correlates, contrast with early reports emphasizing cardiac morbidity and poor prognosis in this syndrome.
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PMID:Cardiac involvement in the Churg-Strauss syndrome. 1667 97

Kawasaki disease is an acute vasculitis of childhood that predominantly affects the coronary arteries. The etiology of Kawasaki disease remains unknown, although an infectious agent is strongly suspected based on clinical and epidemiologic features. A genetic predisposition is also likely, based on varying incidences among ethnic groups, with higher rates in Asians. Symptoms include fever, conjunctival injection, erythema of the lips and oral mucosa, rash, and cervical lymphadenopathy. Some children with Kawasaki disease develop coronary artery aneurysms or ectasia, ischemic heart disease, and sudden death. Kawasaki disease is the leading cause of acquired heart disease among children in developed countries. This article provides a summary of the diagnostic and treatment guidelines published by the American Heart Association.
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PMID:Kawasaki disease: summary of the American Heart Association guidelines. 1703 51

Kawasaki disease is a multi system disorder with varying clinical expressions. This disease is an acute systemic vasculitis of unknown etiology that has recently recognized as a leading cause of acquired heart disease in children of many developed countries. We describe an unusual instance of hair loss in a patient with Kawasaki disease. A 26 months old boy developed prolonged high fever, bilateral conjunctival infection, arthralgia and erythromatosis skin rash. He was admitted to the hospital with the diagnosis of Kawasaki disease. Laboratory results included an erythrocyte sedimentation rate (ESR) above 100 and platelet count > 1000,000. The patient developed acute and unprovoked scalp hair loss. He was treated with intravenous immunoglobulin (IVIG) 2 g/kg and aspirin 100 mg/kg/day with complete improvement of signs and symptoms. This report documents hair loss as an uncommon presentation of Kawasaki disease.
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PMID:Hair loss as a sign of Kawasaki disease. 1723 74

Kawasaki disease is the most common cause of multisystem vasculitis in childhood. Kawasaki disease has been reported throughout the world and affects children of all ethnicity. Coronary artery damage from Kawasaki disease is the leading cause of acquired heart disease in children in the developed world. Diagnostic tests and prognostic markers are lacking, and questions remain unanswered in our understanding of the etiopathogenesis of the disease, thus limiting our ability to improve therapy and coronary outcome. In this article I will review advances made in an animal model of disease, which has helped advance our understanding of the etiology and pathogenesis of this fascinating clinical syndrome.
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PMID:Lessons learned from an animal model of Kawasaki disease. 1742 74

Kawasaki disease (KD) is primarily the systemic vasculitis of childhood that affects mainly the medium-sized arteries, such as the coronary arteries. KD is the leading cause of acquired heart disease, whereas the incidence of rheumatic fever has declined. The most serious complication is coronary artery involvement. Among the children with KD who developed cardiac complications, pericarditis is a rare complication, with an incidence of 0.07%. We report our experience in a 5.5-year-old child with KD complicated with aneurysm of the left anterior descendant coronary artery and septated pericardial effusion, which has not been reported in the literature. The pericardial effusion disappeared very dramatically with intravenous immunoglobulin (IVIG) therapy. We would like to point out that septated pericardial effusion in cases of KD do not need any further therapy other than IVIG and high-dose acetylsalicylic acid.
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PMID:Septated pericarditis associated with Kawasaki disease: a brief case report. 1799 May 88

Kawasaki disease (KD) is a systemic vasculitis condition with a relatively unknown etiology. First described in 1967 by Tomisaku Kawasaki in Japan, KD has come to be widely diagnosed in every region of the world. The disease has a high prevalence in children ages 6 months to 5 years, particularly in those of Japanese descent. Patients often present with a high fever, rash, lymphadenopathy, and conjunctival injections, but there is no diagnostic test for KD. This paper presents data from our Kawasaki registry including 99 patients with emphasis on Kawasaki cardiopathy. Three patients died from complications of KD, and 1 patient underwent heart transplant for massive aneurysmal dilatation. The 4 explanted hearts showed a spectrum of pathological findings (acute thrombosis, vasculitis, and myocarditis), and 1 patient showed the long-term sequelae of vasculitis in the form of massive aneurysmal dilatation. Among the survivors, 30% showed aneurysmal dilatation. This paper reviews the most recent information regarding Kawasaki cardiopathy and underlying molecular mechanisms.
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PMID:Kawasaki disease: four case reports of cardiopathy with an institutional and literature review. 1800 Nov 58

Kawasaki disease is an acute, self-limited vasculitis of infants and children that is now the most common cause of acquired heart disease in the pediatric age group in the United States and Japan. This report presents a case of classic Kawasaki disease with reversible sensorineural hearing loss that was treated with steroid. In conclusion, Kawasaki disease caregivers must be aware of possible sensorineural hearing loss, which is reversible by early intervention and thus improves quality of life.
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PMID:Reversible sensorineural hearing loss in a girl with Kawasaki disease. 1824 48

Kawasaki disease (KD) was considered to be good prognosis in self-limited at first. But, sudden death followed KD has been reported. It became clear that KD produce the peculiar state of myocardial infarction in infants and young children. Now, KD took the place of the rheumatic fever and got the most important disease of acquired heart disease in childhood. Cardiovascular disorder is mainly included with the coronary arterial aneurysm. And aneurysm in an subaxial and iliac artery were complicated. Every organ flame in a whole body was produced with systematic vasculitis mainly on a medium size or small size artery of a whole body. We summarize about complications of a cardiovascular system and the other organs followed KD.
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PMID:[Cardiovascular and the other complication followed with Kawasaki disease]. 1826 Mar 27

Kawasaki disease (KD) is a febrile systemic vasculitis complicated by coronary and peripheral arterial aneurysms in 20% to 35% of untreated patients. It is reported as the commonest cause of acquired heart disease in children in developed countries, and may be a risk for adult ischaemic heart disease. Although KD has been reported all over the world, it is overexpressed among Asian populations, especially Japanese people. The annual incidence for 100,000 children under five years of age is reported to be 8.1 in the United Kingdom, 17.1 in the United States and 112 in Japan. The principal clinical feature is fever, accompanied by extremity changes, polymorphous rash, conjunctival injection, changes in lips and oral cavity, and cervical lymphadenopathy. The disease pathogenesis is still unknown and several theories have been proposed, including the possibility of an infection by a toxin-secreting microorganism and of a superantigen-driven process. Despite numerous efforts there is still no diagnostic test available for KD, and the diagnosis is based on clinical criteria after the exclusion of other diseases presenting with high persistent fever. Prompt diagnosis is critical, since the early administration of intravenous immunoglobulins and aspirin reduces the rate of coronary abnormalities to less than 5% of patients.
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PMID:[Kawasaki disease]. 1832 30

Bartonella species are an important cause of culture-negative endocarditis, with recognized risk factors of alcoholism, homelessness, cat exposure, and pre-existing valvular disease. We report a case of Bartonella henselae endocarditis in a 36-year-old woman with complex congenital heart disease who presented with a 7-month history of hemolytic anemia, leukocytoclastic vasculitis, and recurrent fevers. Transesophageal echocardiogram revealed vegetations on the patient's native aortic valve and in the right ventricular to pulmonary artery conduit and associated bioprosthetic valve. Diagnosis of B. henselae was confirmed with serum antibody and polymerase chain reaction (PCR) testing and tissue stains. The patient was treated successfully with surgical resection and prolonged antimicrobial therapy with ceftriaxone, gentamicin, and doxycycline. A review of the literature suggests prosthetic valves and complex congenital heart disease are risk factors for Bartonella endocarditis, and a high index of suspicion with antibody and PCR testing can expedite diagnosis and improve outcomes.
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PMID:Bartonella endocarditis in complex congenital heart disease. 1837 22


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